Clinical Features of Congenital Pseudarthrosis of the Tibia in Children：a Study Focusing on 514 Cases in China

Abstract Background: Congenital pseudarthrosis of the tibia (CPT) is a refractory and rare disease. Because of its extremely low incidence, little is known about its clinical features. In this retrospective study, the aim of this study was to analyze the clinical characteristics of patients with CPT. Methods: This is a retrospective study of children with CPT identified by radiological review. Investigations of CPT included general condition, the characteristics of CPT, treatment methods and surgical complications. Results: We have collected 514 CPTs from 1999 to March 2020 in our hospital, 317 (61.67%) boys, 197 (38.33%) girls; 330 (62.86%) in Crawford IV; 510 (97.14%) in mid and distal 1/3 tibia; 481 (93.58%) in less than 3 years of first appearance of symptom; 297 (57.78%) in less than 3 years of the first visit of outpatient. The most common postoperative complication was ankle valgus (101, 39.60%), followed by limb length discrepancy（91, 35.69%), refracture (38, 14.90%), osteomyelitis (15, 5.88%) and removal of internal fixation (10,3.93%) . Conclusions: CPT with higher incidence of Crawford IV frequently occurs in boys and middle or distal part of the tibia; most patients have the first appearance of symptom and the first visit of outpatient before 3 years; the major surgical complications were ankle valgus and limb length discrepancy.

Download Full-text

Efficacy of the “Eiffel tower” double titanium elastic nailing in combined management of congenital pseudarthrosis of the tibia: preliminary outcomes of 17 cases with review of literature

BMC Musculoskeletal Disorders ◽

10.1186/s12891-021-04382-7 ◽

2021 ◽

Vol 22 (1) ◽

Author(s):

Xiaoyu Wang ◽

Li Shi ◽

Rui Zhang ◽

Wenbo Wang ◽

Feng Wang ◽

...

Keyword(s):

Bone Grafting ◽

Limb Length Discrepancy ◽

Limb Length ◽

Bone Union ◽

Length Discrepancy ◽

Congenital Pseudarthrosis ◽

The Mean ◽

Primary Union ◽

Ilizarov Fixation

Abstract Background Difficulty in obtaining union, recurrent fractures, and residual deformities remain the problems challenging the management of congenital pseudarthrosis of the tibia (CPT). We applied the “Eiffel Tower” double titanium elastic nails (TENs) in the existing combined approach, which takes advantages of TEN’s mechanical stability with the protection against refracture, Ilizarov’s high fusion rate with alignment control and the biologic environment provided by bone grafting for bony union. The results of this procedure are presented and discussed. Methods Seventeen patients with CPT treated by combined surgery including pseudarthrosis resection, the “Eiffel Tower” double TENs technique, autogenous iliac bone grafting, and Ilizarov fixation between 2013 and 2019 were retrospectively investigated. Signs of bone union, limb length discrepancy (LLD), rate of refracture, and degree of residual deformities were reviewed. The AOFAS Ankle Hindfoot scale and measurement of ankle motion were used to evaluate ankle function. The mean follow-up time was 40.5 (11 to 91) months. Results The mean age at index surgery was 6.2 (2.5 to 15) years. Union of the pseudarthrosis was achieved in 100% of cases. Among them, 15 (88.2%) patients obtained union of the pseudarthrosis on the first attempt (primary union). The average time to primary union was 3.8 (2 to 6) months. The rest 2 cases achieved union after additional surgeries (secondary union). In terms of complications, refracture occurred in 2 patients (11.8%) and 4 patients (23.5%) developed pin infection. The mean limb length discrepancy at the final follow up was 33.4 (6–141) mm. The average AOFAS score improved from 38.2 (27 to 51) pre-operatively to 77 (63 to 87) post-operatively (p < 0.01). Conclusions The “Eiffel Tower” double TENs technique is an ideal intramedullary fixation method in the surgical treatment of CPT. The combination of TENs technique with bone grafting and Ilizarov fixation has the advantages of early bone union, less injury on metaphysis, and early functional recovery. Level of evidence Level IV.

Download Full-text

Treatment outcomes of congenital pseudarthrosis of the tibia at Beit Cure International Hospital in Blantyre, Malawi

East and Central African Journal of Surgery ◽

10.4314/ecajs.v23i3.5 ◽

2019 ◽

Vol 23 (3) ◽

pp. 109-118

Author(s):

Inyas L. Akaro ◽

Kyle James ◽

Linda Chokotho ◽

David Burgess ◽

Nyengo Mkandawire ◽

...

Keyword(s):

Treatment Outcomes ◽

Rare Condition ◽

Limb Length Discrepancy ◽

Limb Length ◽

Treatment Modalities ◽

Foot And Ankle ◽

Cross Sectional ◽

Length Discrepancy ◽

Congenital Pseudarthrosis ◽

Intramedullary Rodding

Background: Congenital pseudarthrosis of the tibia (CPT) is a rare condition. The natural history of CPT includes persistent instability and progressive deformity. Several CPT treatment methods have been practiced, however, in Africa where there is scarce information on the modalities of treatment available and their outcomes. Methods: A retrospective cross-sectional study which was conducted among patients with CPT at Beit Cure International Hospital (BCIH), Malawi. Forty-four patients were recruited in this study and their treatment modalities and outcomes were analyzed. Results: Out of 44 patients recruited in this study, majority (63.6%) were male. The majority of cases were stage 4 congenital tibia pseudarthrosis by Crawford classification. Most patients were treated by more than one surgical modality; however, surgical excision and intramedullary rodding was commonly used (54.7%). The outcomes of treatment were good in 5%, fair in 30%, with amputation in 45% and poor outcomes in 20% of the patients. Complications developed in 60% of patients, predominated by limb length discrepancy. The foot and ankle status were rated by Oxford Foot and Ankle scoring system (OxFAQ). Conclusions: Congenital pseudarthrosis of the tibia is a complex congenital disorder with multiple modalities of treatment. Majority of the patients were treated by more than one operation. Some patients ended up with amputation or poor outcome. Limb length discrepancy, deep infection and pin tract infection are among the common complications. Keywords: congenital pseudarthrosis; tibia; treatment outcomes; Malawi

Download Full-text

Intramedullary Bone Lengthening Following Preceding Hip Surgery—A Case Series

Journal of Clinical Medicine ◽

10.3390/jcm9124104 ◽

2020 ◽

Vol 9 (12) ◽

pp. 4104

Author(s):

Lukas Zak ◽

Thomas Manfred Tiefenboeck ◽

Gerald Eliot Wozasek

Keyword(s):

Hip Dysplasia ◽

Young Patients ◽

Limb Lengthening ◽

Limb Length Discrepancy ◽

Limb Length ◽

Hip Surgery ◽

Bone Lengthening ◽

Safe Procedure ◽

Length Discrepancy ◽

Healing Index

Limb length discrepancy (LLD) is a common problem after joint-preserving hip surgeries, hip dysplasia, and hip deformities. Limping, pain, sciatica, paresthesia, and hip instability are common clinical findings and may necessitate limb-lengthening procedures. The study included five patients (two female and three male, mean age of 28 years (20–49; SD: 12)) with symptomatic limb length discrepancy greater than 2.5 cm (mean: 3.6 cm) after total hip arthroplasty (THA), hip dysplasia, or post-traumatic hip surgery. They underwent either ipsi- or contralateral intramedullary limb-lengthening surgeries using the PRECICE™ telescopic nail. All patients achieved complete bone healing and correction of the pelvic obliquity after intramedullary lengthening. None of the patients had a loss of proximal or distal joint motion. The mean distraction-consolidation time (DCT) was 3.8 months, the distraction index (DI) 0.7 mm/day, the lengthening index (LI) 1.8 months/cm, the consolidation index (CI) 49.2 days/cm, the healing index (HI) 1.1 months/cm, and the modified healing index (HI*) 34 days/cm. Intramedullary limb lengthening after LLD in cases of hip dysplasia, hip deformity, and various kinds of hip surgery is a useful and safe procedure in young patients to achieve equal limb length. No functional impairment of the preceded hip surgery was seen.

Download Full-text